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Noncompaction Syndrome of the Myocardium: Pathophysiology and Imaging Pearls

Zenooz, Navid A. MD*; Zahka, Keneth G. MD; Siwik, Ernest S. MD; Gilkeson, Robert C. MD

doi: 10.1097/RTI.0b013e31819ca93e
Pictorial Essay

Patients with noncompaction syndrome of the myocardium may present with cardiac arrhythmia, thromboembolic events, or left ventricular failure. Associations with other diseases, including neurologic or other cardiac syndromes, have been noted. Noncompaction syndrome of the myocardium is primarily diagnosed by echocardiography; however, CT scanning and magnetic resonance imaging are useful tools for determining severity and the patient's prognosis. With these methods, high-resolution images of the myocardium are obtained that enable better recognition of the areas over which the trabeculae are distributed. Early diagnosis can improve the patient's survival by premature heart transplantation or implantation of a defibrillator. Also, as there are many reports of occurrence of this syndrome in several members of the same family (mainly X-linked inheritance), upon accurate and early diagnosis, the patient's family can be further screened.

*Department of Radiology, Yale New Haven Hospital, New Haven, CT

Departments of Pediatric Cardiology and

Radiology, University Medical Center, Case Western Reserve University, Cleveland, OH

Reprints: Navid A. Zenooz, MD, Department of Radiology, Yale New Haven Hospital, 20 York Street, New Haven, CT 06519 (e-mail: znavid@yahoo.com).

© 2010 Lippincott Williams & Wilkins, Inc.